title: Insight into congenital absence of the portal vein: is it rare?
abstract: Congenital absence of portal vein (CAPV) was a rare event in the past. However, the number of detected CAPV cases has increased in recent years because of advances in imaging techniques. Patients with CAPV present with portal hypertension (PH) or portosystemic encephalopathy (PSE), but these conditions rarely occur until the patients grow up or become old. The patients usually visit doctors for the complications of venous shunts, hepatic or cardiac abnormalities detected by ultrasonography (US), computed tomography (CT) and magnetic resonance imaging (MRI). The etiology of this disease is not clear, but most investigators consider that it is associated with abnormal embryologic development of the portal vein. Usually, surgical intervention can relieve the symptoms and prevent occurrence of complications in CAPV patients. Moreover, its management should be stressed on a case-by-case basis, depending on the type or anatomy of the disease, as well as the symptoms and clinical conditions of the patient.
authors.affiliation: Department of General Surgery, Sir Run Run Shaw Hospital, Medical College of Zhejiang University, Hangzhou 310016, Zhejiang Province, China.
authors.forename: Guo-Hua
authors.initials: GH
authors.lastname: Hu
authors.forename: Lai-Gen
authors.initials: LG
authors.lastname: Shen
authors.forename: Jin
authors.initials: J
authors.lastname: Yang
authors.forename: Jin-Hua
authors.initials: JH
authors.lastname: Mei
authors.forename: Yue-Feng
authors.initials: YF
authors.lastname: Zhu
mesh_terms: D006501:Hepatic Encephalopathy / Q000209:etiology; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology; D015994:Incidence; D011169:Portal Vein / Q000002:abnormalities* / Q000196:embryology; D054079:Vascular Malformations / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology*